Large Cell Neuroendocrine Cancer (LCNEC) of Uterine Cervix (original) (raw)
Large-cell neuroendocrine carcinoma of the uterine cervix--a clinicopathological study of five cases
PubMed, 2001
Objective: The present study describes 5 cases of large-cell neuroendocrine carcinoma (LCNEC) of the uterine cervix, evaluating their clinical features and pathological profiles. Methods: Clinical data were obtained from the patients' clinical files at the combined gynaecological-oncology unit of Johannesburg Hospital and the University of the Witwatersrand Medical School, Johannesburg, South Africa. A histopathological diagnosis was obtained after biopsy material from all 5 patients was examined microscopically and subjected to immunohistochemical staining with MNF116 (pankeratin) synaptophysin and chromagranin A, all of which are neuroendocrine markers. Two patients received pelvic radiotherapy only. None of the 5 patients in this series received chemotherapy or underwent surgery. Results: All 5 patients were adult females, with an average age of 57.3 years. The majority were multiparous, with the most common presenting complaint being vaginal bleeding. Three of the 5 patients presented with advanced-stage cervical carcinoma, with evidence of metastases in 2 of them. Treatment responses and long-term survival in our series proved to be disappointing as 3 of the 5 patients died in less than 6 months. On histopathological examination, all 5 tumours showed features of a high-grade poorly differentiated malignant neoplasm with ulceration and extensive tumour necrosis including trabecular and organoid growth patterns. All 5 neoplasms also showed strong immunoreactivity for MNF116, while their endocrine nature was confirmed by staining for synaptophysin in all cases. None of the tumours showed positive straining for chromagranin A. Conclusions: LCNECs are rare tumours and distinct from other neoplasms of the uterine cervix. The results of this study reaffirm the biologically aggressive nature of this uncommon tumour and its very unfavourable prognosis.
Gynecologic large cell neuroendocrine carcinoma: A review
Rare Tumors
Large cell neuroendocrine carcinomas (LCNEC) are rare, aggressive high-grade neuroendocrine neoplasms within the neuroendocrine cell lineage spectrum. This manuscript provides a detailed review of published literature on LCNEC of gynecological origin. We performed a PubMed search for material available on gynecologic LCNEC. We analyzed 104 unique cases of gynecologic LCNECs, of which 45 were cervical primary, 45 were ovarian, 13 were uterine, and 1 was vaginal. A total of 45 cases of cervical LCNEC were identified with a median age of 36 years. Median overall survival was 16 months. We identified 45 ovarian LCNEC cases in the published literature with a median age of 54 years. Median overall survival was 8 months. 13 LCNEC cases of uterine origin were identified; 12 out of 13 were of endometrial origin and the median age was 71 years. The majority of patients presented with Stage III/IV disease (stages I–IV were 31%, 8%, 38%, and 23%, respectively). Gynecologic LCNEC is an aggressiv...
Annals of Diagnostic Pathology, 2013
Neuroendocrine carcinoma cervix Small cell carcinoma cervix Large cell neuroendocrine carcinoma cervix Immunohistochemistry in cervix carcinomas Cervix cancer Neuroendocrine carcinomas of the cervix are uncommon, characterized by a histomorphological spectrum and, mostly, an aggressive clinical course. There are only few substantial studies on such cases documented from our country, where cervical cancer is the second most common cancer affecting women. Herein, we present a spectrum of 50 cervical neuroendocrine carcinomas, including histopathologic features, terminology, immunohistochemical (IHC) profile, and clinical outcomes, wherever available. Fifty tumors occurred in women, with their age ranging from 23 to 69 years (mean, 48.6 years; median, 46.5 years). Stagewise, among 25 cases, most cases (6, or 24%) presented with stage IB. Average tumor size was 4.7 cm. On histopathologic review, 26 tumors (52%) were classified as small cell carcinoma (SMCA); 14 (28%), as large cell neuroendocrine carcinomas (LCNECs); 4 (8%), as SMCA + LCNECs; and 6, as mixed carcinomas, including 3 tumors (6%) with SMCA and squamous cell carcinoma (SCC), 2 tumors (4%) with LCNEC and adenocarcinoma, and a single tumor (2%) with LCNEC and squamous cell carcinoma. On IHC performed in 41 tumors (82%), 36 tumors (87.8%) were positive for at least a single neuroendocrine marker, and 22 (53.6%) expressed 2 neuroendocrine markers. Synaptophysin was positive in 22 (59.4%) of 37 tumors; chromogranin, in 27 (72.9%) of 37; CD56, in 8 (100%) of 8; and neuron-specific enolase in 7 (87.5%) of 8 tumors. Treatment wise, among 30 patients (60%), 6 (20%) underwent surgery, including Wertheim hysterectomy (5) and simple hysterectomy (1); 8 (26.6%) underwent surgery with adjuvant treatment, and 10 patients (33.3%) were offered chemotherapy and/or radiotherapy. On follow-up (27 patients, or 54%) over 1 to 144 months, 16 patients (59.2%) were alive with disease over median duration of 9 months, and 7 (25.9%) were free of disease over median duration of 26.5 months. There were 5 recorded deaths. Thirteen tumors (48.1%) metastasized, most commonly to liver. In cases with early stage disease and adjuvant treatment, including radiotherapy, LCNEC histology fared well. This study forms the largest documented series on cervical neuroendocrine carcinomas from our country, testifying the current histopathologic classification system. Although SMCAs can be recognized on morphology, LCNECs need to be correctly identified because these can be misdiagnosed in the absence of neuroendocrine markers. Synaptophysin, chromogranin, and CD56 are optimal IHC markers. Small cell carcinomas, pure or mixed, are relatively more aggressive. All these tumors are best treated with multimodal therapy. Early stage disease treated with radical surgery and adjuvant treatment seems to increase survival. Despite aggressive treatment, prognosis is dismal.
2019
Large cell neuroendocrine carcinoma (LCNEC) of the endometrium is a rare and highly malignant neoplasm with no characteristic findings in terms of clinical manifestations, diagnostic imaging, or pathology, and thus, the definitive preoperative diagnosis of LCNEC is difficult. A 61-year-old postmenopausal woman presented with low abdominal pain and a rapidly growing uterine mass without postmenopausal bleeding. Magnetic resonance imaging of the pelvis revealed an enlarged uterus with a 7.5 cm mass. Intraoperative frozen examination revealed a malignant tumor, and accordingly, cytoreductive surgery was performed. Microscopically, the tumor showed extensive necrosis, hemorrhage, and an organoid nesting pattern of large cells. Immunohistochemistry revealed tumor cells were diffusely positive for the neuroendocrine markers CD56 and synaptophysin. Thus the tumor was diagnosed as LCNEC of endometrium. Postoperatively, the disease pursued a progressive course and relapsed even after repeate...
Journal of clinical and diagnostic research : JCDR, 2016
Ovarian Large Cell Neuroendocrine Carcinoma (LCNEC) is a recently described rare entity, which even more rarely occurs in a 'pure' form without any associated surface epithelial-stromal or germ cell component. Cervix metastasis of ovarian LCNEC has not been reported previously. We report here a case of ovarian LCNEC in a 35-year-old female who presented with abdominal pain and amenorrhea. Grossly the left ovary showed a solid cystic tumour measuring 6 cm in diameter. Histological examination showed a pure LCNEC without any associated component, confirmed by immunohistochemistry. Metastatic tumour deposits with numerous lymphovascular emboli were identified in the cervix. A comprehensive review of literature along with the various differential diagnosis is discussed.
Neuroendocrine carcinoma of cervix and review literature
Neuroendocrine carcinoma of the cervix (NECC) is a rare variant of cervical cancer with poor prognosis and high mortality. Recurrence is seen with multi-organ metastasis including liver. Case presentation: A 65 year old female presented with vaginal bleeding for the past one year. Cervical cancer screening and biopsy demonstrated poorly differentiated squamous carcinoma. Immunohistochemistry showed positive expression of chromogranin, synaptophysin, pancytokeratin, TTP1, and CEA and negative expression of p40 and estrogen receptors. An adenocarcinoma with neuroendocrine tumor was suggested. Hysterectomy with bilateral salpingo-oophrectomy was performed. This was followed by carboplatin and etoposide therapy to have clinical remission for a year. Then recurrence was observed to start same drugs again resulting in to partial improvement. It was followed by radiotherapy. The patient succumbed to death approximately after three months. Conclusion: A metastatic lesion in liver may be a case of Neuroendocrine tumor of cervix, a rare condition that can be easily missed on histopathological examination. More studies are required to establish a standard therapeutic protocol.
A Rare Case of Neuroendocrine Tumour of Cervix: A Case Report
Indian Journal of Gynecologic Oncology, 2018
Neuroendocrine tumours of cervix are rare variety of carcinoma cervix. They have aggressive behaviour and poor outcome in most of the cases. They account for around 0.4% of all cancers of cervix. Rarity of this tumour makes it difficult to establish a management protocol. Here we report such a case where a different concurrent chemoradiotherapy plan was followed and patient showed good response.